Characterization of B cells in muscle-specific kinase antibody myasthenia gravis

Neurology. Neuroimmunology and Neuroinflammation
Jeffrey T GuptillKent J Weinhold

Abstract

To characterize B-cell subsets in patients with muscle-specific tyrosine kinase (MuSK) myasthenia gravis (MG). In accordance with Human Immunology Project Consortium guidelines, we performed polychromatic flow cytometry and ELISA assays in peripheral blood samples from 18 patients with MuSK MG and 9 healthy controls. To complement a B-cell phenotype assay that evaluated maturational subsets, we measured B10 cell percentages, plasma B cell-activating factor (BAFF) levels, and MuSK antibody titers. Immunologic variables were compared with healthy controls and clinical outcome measures. As expected, patients treated with rituximab had high percentages of transitional B cells and plasmablasts and thus were excluded from subsequent analysis. The remaining patients with MuSK MG and controls had similar percentages of total B cells and naïve, memory, isotype-switched, plasmablast, and transitional B-cell subsets. However, patients with MuSK MG had higher BAFF levels and lower percentages of B10 cells. In addition, we observed an increase in MuSK antibody levels with more severe disease. We found prominent B-cell pathology in the distinct form of MG with MuSK autoantibodies. Increased BAFF levels have been described in other autoimmune...Continue Reading

Citations

Mar 6, 2016·Journal of Neuroimmunology·Tanel PungaAnna Rostedt Punga
Oct 30, 2015·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Jeffrey T GuptillMatthew N Meriggioli
Oct 23, 2015·Muscle & Nerve·John S YiJeffrey T Guptill
Feb 7, 2017·Muscle & Nerve·Rup TandanDiantha B Howard
Sep 25, 2017·Muscle & Nerve·John S YiKevin C O'Connor
Mar 29, 2019·Journal of Neurology·Nishita Singh, Vinay Goyal
Dec 4, 2019·Continuum : Lifelong Learning in Neurology·Emma Ciafaloni
Jun 28, 2017·Current Opinion in Neurology·Amelia Evoli
Mar 8, 2017·Frontiers in Neurology·Md Rezaul KarimYun-Fu Wang
Jan 30, 2018·Annals of the New York Academy of Sciences·Maartje G HuijbersJan J Verschuuren
Jan 31, 2018·Annals of the New York Academy of Sciences·Panos StathopoulosKevin C O'Connor
Jan 26, 2018·Annals of the New York Academy of Sciences·Mariapaola MarinoAmelia Evoli
Nov 28, 2018·Annals of Clinical and Translational Neurology·Vuslat YilmazRozen Le Panse
Dec 22, 2017·Annals of the New York Academy of Sciences·Amelia EvoliMariapaola Marino
Apr 27, 2016·Journal of Neuroscience Research·Jinming HanTao Jin
Jun 22, 2018·Annals of Clinical and Translational Neurology·Elena Cortés-VicenteIsabel Illa
Jan 2, 2021·Frontiers in Neurology·Ying WuOliver A Garden
Nov 14, 2020·Clinical and Experimental Immunology·A UzawaK Utsugisawa
May 28, 2021·Expert Review of Clinical Immunology·Amelia EvoliValentina Damato
Aug 3, 2021·Archivum Immunologiae Et Therapiae Experimentalis·Ewa SobieszczukAnna Kostera-Pruszczyk
Sep 26, 2021·Neurology. Neuroimmunology and Neuroinflammation·Yohei YamamotoYuishin Izumi

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Methods Mentioned

BETA
blood draw
flow cytometry
ELISA
PMA
immunoprecipitation

Software Mentioned

Prism Pad
Flowjo

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